{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,8]],"date-time":"2026-01-08T02:36:38Z","timestamp":1767839798769,"version":"3.49.0"},"reference-count":58,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2018,6,10]],"date-time":"2018-06-10T00:00:00Z","timestamp":1528588800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["81601099"],"award-info":[{"award-number":["81601099"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Neuropathology Appl Neurobio"],"published-print":{"date-parts":[[2019,4]]},"abstract":"<jats:sec><jats:title>Aims<\/jats:title><jats:p>Quantitative estimation of cortical neurone loss in cases with chorea\u2010acanthocytosis (ChAc) and its impact on laminar composition.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We used unbiased stereological tools to estimate the degree of cortical pathology in serial gallocyanin\u2010stained brain sections through the complete hemispheres of three subjects with genetically verified ChAc and a range of disease durations. We compared these results with our previous data of five Huntington's disease (HD) and five control cases. Pathoarchitectonic changes were exemplarily documented in TE1 of a 61\u2010year\u2010old female HD\u2010, a 60\u2010year\u2010old female control case, and ChAc3.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Macroscopically, the cortical volume of our ChAc cases (ChAc1\u20133) remained close to normal. However, the average number of neurones was reduced by 46% in ChAc and by 33% in HD (<jats:italic>P<\/jats:italic> = 0.03 for ChAc &amp; HD <jats:italic>vs<\/jats:italic>. controls; <jats:italic>P<\/jats:italic> = 0.64 for ChAc <jats:italic>vs<\/jats:italic>. HD). Terminal HD cases featured selective laminar neurone loss with pallor of layers III, V and VIa, a high density of small, pale, closely packed radial fibres in deep cortical layers VI and V, shrinkage, and chromophilia of subcortical white matter. In ChAc, pronounced diffuse astrogliosis blurred the laminar borders, thus masking the complete and partial loss of pyramidal cells in layer IIIc and of neurones in layers III, V and VI.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>ChAc is a neurodegenerative disease with distinct cortical neurodegeneration. The hypertrophy of the peripheral neuropil space of minicolumns with coarse vertical striation was characteristic of ChAc. The role of astroglia in the pathogenesis of this disorder remains to be elucidated.<\/jats:p><\/jats:sec>","DOI":"10.1111\/nan.12495","type":"journal-article","created":{"date-parts":[[2018,5,3]],"date-time":"2018-05-03T03:48:07Z","timestamp":1525319287000},"page":"230-243","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Pathoarchitectonics of the cerebral cortex in chorea\u2010acanthocytosis and Huntington's disease"],"prefix":"10.1111","volume":"45","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6150-8305","authenticated-orcid":false,"given":"J.","family":"Liu","sequence":"first","affiliation":[{"name":"Department of Neurology Xuanwu Hospital Capital Medical University Beijing China"},{"name":"Neurologische Klinik und Poliklinik Ludwig\u2010Maximilians\u2010Universit\u00e4t M\u00fcnchen M\u00fcnchen Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H.","family":"Heinsen","sequence":"additional","affiliation":[{"name":"Department of Psychiatry, Psychosomatics and Psychotherapy Center of Mental Health University Hospital W\u00fcrzburg W\u00fcrzburg Germany"},{"name":"Ageing Brain Study Group Department of Pathology University of S\u00e3o Paulo Medical School S\u00e3o Paulo Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L. T.","family":"Grinberg","sequence":"additional","affiliation":[{"name":"Department of Neurology University of California, San Francisco San Francisco CA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E.","family":"Alho","sequence":"additional","affiliation":[{"name":"Pra\u00e7a Amadeu Amaral S\u00e3o Paulo Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"suffix":"Jr","given":"E.","family":"Amaro","sequence":"additional","affiliation":[{"name":"Department of Radiology University of S\u00e3o Paulo Medical School S\u00e3o Paulo Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C. A.","family":"Pasqualucci","sequence":"additional","affiliation":[{"name":"Ageing Brain Study Group Department of Pathology University of S\u00e3o Paulo Medical School S\u00e3o Paulo Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"U.","family":"R\u00fcb","sequence":"additional","affiliation":[{"name":"Experimental Neurobiology (Anatomical Institute II) Goethe\u2010University Frankfurt\/Main Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"K.","family":"Seidel","sequence":"additional","affiliation":[{"name":"Experimental Neurobiology (Anatomical Institute II) Goethe\u2010University Frankfurt\/Main Germany"},{"name":"Anatomy &amp; Cell Biology Medical Faculty Anatomical Institute University of Bonn Bonn Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"W.","family":"den Dunnen","sequence":"additional","affiliation":[{"name":"Department of Pathology and Medical Biology University Medical Center Groningen University of Groningen Groningen The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"T.","family":"Arzberger","sequence":"additional","affiliation":[{"name":"Center for Neuropathology and Prion Research Ludwig\u2010Maximilians\u2010University Munich Munich Germany"},{"name":"Department of Psychiatry and Psychotherapy Ludwig\u2010Maximilians\u2010University Munich Munich Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.","family":"Schmitz","sequence":"additional","affiliation":[{"name":"Department of Neuroanatomy Ludwig\u2010Maximilians\u2010Universit\u00e4t M\u00fcnchen Munich Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. C.","family":"Kiessling","sequence":"additional","affiliation":[{"name":"Department of Neuroanatomy Ludwig\u2010Maximilians\u2010Universit\u00e4t M\u00fcnchen Munich Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"B.","family":"Bader","sequence":"additional","affiliation":[{"name":"Neurologische Klinik und Poliklinik Ludwig\u2010Maximilians\u2010Universit\u00e4t M\u00fcnchen M\u00fcnchen Germany"},{"name":"Clienia Privatklinik f\u00fcr Psychiatrie und Psychotherapie Oetwil am See Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8857-5383","authenticated-orcid":false,"given":"A.","family":"Danek","sequence":"additional","affiliation":[{"name":"Neurologische Klinik und Poliklinik Ludwig\u2010Maximilians\u2010Universit\u00e4t M\u00fcnchen M\u00fcnchen Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2018,6,10]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-540-71693-8"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/88821"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.ejhg.5200866"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/88825"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410030312"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine1962.23.118"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100029516"},{"key":"e_1_2_9_9_1","first-page":"13","article-title":"Neuroacanthocytosis. A clinical, haematological and pathological study of 19 cases","volume":"114","author":"Hardie RJ","year":"1991","journal-title":"Brain"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.870090303"},{"key":"e_1_2_9_11_1","first-page":"178","article-title":"Neuroacanthocytosis. Review of literature and case report","volume":"34","author":"Rafalowska J","year":"1996","journal-title":"Folia Neuropathol"},{"key":"e_1_2_9_12_1","first-page":"77","article-title":"Chorea\u2010acanthocytosis: neuropathology of brain and peripheral nerve","volume":"21","author":"Vital A","year":"2002","journal-title":"Clin Neuropathol"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-540-71693-8_15"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-008-0403-1"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-009-0617-x"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-013-1241-3"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/nan.12403"},{"key":"e_1_2_9_18_1","first-page":"33","article-title":"Neurodegenerative disorders with extrapyramidal features \u2013 a neuropathological overview","volume":"46","author":"Jellinger KA","year":"1995","journal-title":"J Neural Transm Suppl"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.parkreldis.2018.01.009"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00310376"},{"key":"e_1_2_9_21_1","volume-title":"Vergleichende Lokalisationslehre der Gro\u00dfhirnrinde","author":"Brodmann K","year":"1909"},{"key":"e_1_2_9_22_1","volume-title":"Die Cytoarchitekonik der Hirnrinde des erwachsenen Menschen","author":"von Economo C","year":"1925"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-012-1000-x"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.10676"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20248"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0891-0618(00)00067-3"},{"key":"e_1_2_9_27_1","doi-asserted-by":"publisher","DOI":"10.1179\/his.1991.14.3.167"},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awl386"},{"key":"e_1_2_9_29_1","doi-asserted-by":"publisher","DOI":"10.1002\/ar.1092310411"},{"key":"e_1_2_9_30_1","doi-asserted-by":"publisher","DOI":"10.3389\/fnana.2014.00027"},{"key":"e_1_2_9_31_1","volume-title":"Cellular structure of the human cerebral cortex","author":"von Economo C","year":"2009"},{"key":"e_1_2_9_32_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1018506113256"},{"key":"e_1_2_9_33_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004290050191"},{"key":"e_1_2_9_34_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004010051038"},{"key":"e_1_2_9_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0891-0618(00)00066-1"},{"key":"e_1_2_9_36_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0891-0618(00)00085-5"},{"key":"e_1_2_9_37_1","first-page":"185","article-title":"An argyrophil III method for the demonstration of fibrous neuroglia","volume":"29","author":"Gallyas F","year":"1981","journal-title":"Acta Morphol Acad Sci Hung"},{"key":"e_1_2_9_38_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-319-19285-7_1"},{"key":"e_1_2_9_39_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2004.11.024"},{"key":"e_1_2_9_40_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000416389.94466.01"},{"key":"e_1_2_9_41_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2990.1992.tb00824.x"},{"key":"e_1_2_9_42_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0079-6123(02)36039-4"},{"key":"e_1_2_9_43_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awf110"},{"key":"e_1_2_9_44_1","doi-asserted-by":"publisher","DOI":"10.3389\/fnana.2012.00022"},{"key":"e_1_2_9_45_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.10226"},{"key":"e_1_2_9_46_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awv110"},{"key":"e_1_2_9_47_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.psychres.2008.04.007"},{"key":"e_1_2_9_48_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-006-0085-5"},{"key":"e_1_2_9_49_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.58.3.428"},{"key":"e_1_2_9_50_1","doi-asserted-by":"publisher","DOI":"10.1177\/088307380201700211"},{"key":"e_1_2_9_51_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.060009897"},{"key":"e_1_2_9_52_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-005-0014-z"},{"key":"e_1_2_9_53_1","doi-asserted-by":"crossref","first-page":"45","DOI":"10.3233\/JHD-140092","article-title":"Widespread heterogeneous neuronal loss across the cerebral cortex in Huntington's disease","volume":"3","author":"Nana AL","year":"2014","journal-title":"J Huntingtons Dis"},{"key":"e_1_2_9_54_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.24162"},{"key":"e_1_2_9_55_1","first-page":"631","article-title":"Zur Lehre der Erkrankungen des stri\u00e4ren Systems","volume":"25","author":"Vogt C","year":"1920","journal-title":"J Psychol Neurol"},{"key":"e_1_2_9_56_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410200613"},{"key":"e_1_2_9_57_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005072-199902000-00006"},{"key":"e_1_2_9_58_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2990.1995.tb01024.x"},{"key":"e_1_2_9_59_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrn3469"}],"container-title":["Neuropathology and Applied Neurobiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fnan.12495","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/nan.12495","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/nan.12495","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/nan.12495","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,10]],"date-time":"2023-09-10T21:52:02Z","timestamp":1694382722000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/nan.12495"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,6,10]]},"references-count":58,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2019,4]]}},"alternative-id":["10.1111\/nan.12495"],"URL":"https:\/\/doi.org\/10.1111\/nan.12495","archive":["Portico"],"relation":{},"ISSN":["0305-1846","1365-2990"],"issn-type":[{"value":"0305-1846","type":"print"},{"value":"1365-2990","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,6,10]]},"assertion":[{"value":"2017-07-24","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-02-27","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-06-10","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}